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Congenital constriction band syndrome
Journal of Pediatric Orthopedics
|November 1, 1984
Summary
Congenital constriction band syndrome primarily affects limb extremities, causing bands, amputations, and syndactyly. Some clubfoot cases result from nerve compression by deep bands.
Area of Science:
- Orthopedics
- Genetics
- Developmental Biology
Background:
- Congenital constriction band syndrome (CCBS) is a rare condition affecting limb development.
- Manifestations include limb constriction, amputation, and syndactyly, with variable severity and distribution.
Purpose of the Study:
- To analyze clinical manifestations and associated anomalies in CCBS patients.
- To determine the distribution and patterns of limb involvement in CCBS.
- To investigate the etiology of associated clubfoot deformities in CCBS.
Main Methods:
- Retrospective review of 83 patients diagnosed with CCBS.
- Analysis of clinical data, including type and location of anomalies.
- Detailed examination of associated anomalies, particularly clubfoot, and nerve status.
Main Results:
- Constriction bands, amputation, and acrosyndactyly were the most common findings.
- Distal limb involvement was most frequent, with central digits affected more than thumbs.
- Of 19 cases with clubfoot, 10 had paralytic clubfoot due to peroneal nerve compression by bands, while 9 had normal peripheral nerves.
Conclusions:
- CCBS predominantly affects distal extremities, with specific patterns of hand and foot involvement.
- Deep constriction bands can cause secondary paralytic clubfoot through peroneal nerve compression.
- Understanding these patterns aids in diagnosis and management of CCBS and associated anomalies.